Sub-optimal parenting is associated with schizotypic and anxiety personality traits in adulthood.

Part of the variation in personality characteristics has been attributed to the child-parent interaction and sub-optimal parenting has been associated with psychiatric morbidity. In the present study, an extensive battery of personality scales (Trait Anxiety Inventory, Behavioural Inhibition/Activation System questionnaire, Eysenck Personality Questionnaire-Revised, Temperament and Character Inventory, Schizotypal Traits Questionnaire, Toronto Alexithymia Scale) and the Parental Bonding Instrument (PBI) were administered in 324 adult healthy males to elucidate the effects of parenting on personality configuration. Personality variables were analysed using Principal Component Analysis (PCA) and the factors "Schizotypy", "Anxiety", "Behavioural activation", "Novelty seeking" and "Reward dependence" were extracted. Associations between personality factors with PBI "care" and "overprotection" scores were examined with regression analyses. Subjects were divided into "parental style" groups and personality factors were subjected to categorical analyses. "Schizotypy" and "Anxiety" were significantly predicted by high maternal overprotection and low paternal care. In addition, the Affectionless control group (low care/high overprotection) had higher "Schizotypy" and "Anxiety" compared with the Optimal Parenting group (high care/low overprotection). These results further validate sub-optimal parenting as an important environmental exposure and extend our understanding on the mechanisms by which it increases risk for psychiatric morbidity.

Proteolytic matrix metallopeptidases and inhibitors in BCR-ABL1-negative myeloproliferative neoplasms: correlation with JAK2 mutation status.

BACKGROUND/AIMS: Philadelphia chromosome-negative myeloproliferative neoplasms (MPNs) share the same acquired lesion JAK2(V617F) and may exhibit substantial overlap. Variability in JAK activation and allele burden, complemented by host, genetic and non-genetic modifiers, determine the phenotype. The aim of this study was to investigate the presence of the JAK2 mutation in association with the ratio of metallopeptidases inhibitors (TIMPs) to tissue metallopeptidases (MMPs) in MPNs, where inhibitory rather than proteolytic activity in marrow microenvironment appears to predominate. METHODS: 94 patients with polycythemia vera, essential thrombocythemia and primary myelofibrosis, and 102 healthy individuals were evaluated. Allele-specific PCR and RFLP were used to detect JAK2 and genomic status. Serum concentrations of MMP and TIMP were measured by ELISA. The parameters were assessed with covariance analysis, and adjusted for gender, age and co-morbidity. RESULTS: Mutation frequency was 81.91%. Abnormal TIMP/MMP ratios were identified in all three diseases. JAK2 mutation was correlated with significant changes in TIMP concentrations. CONCLUSIONS: Identification of an abnormal TIMP/MMP ratio in all three diseases, regardless of the JAK2 status, indicates invariable marrow remodeling. In this particular group of patients, presence of a JAK2(V617F) mutation, being associated with even higher ratios, appears to be a concurring participant in bone marrow-reforming processes. Additional research may delineate correlates with the JAK2 allelic burden.

Kinetics, function and bone marrow trafficking of CD4+CD25+FOXP3+ regulatory T cells in myelodysplastic syndromes (MDS).

CD4(+)CD25(+)FOXP3(+) T regulatory cells (T(regs)) prevent autoimmunity by restricting overexuberant immune responses, but the same subpopulation can incur detrimental effects on antitumor responses. In both cases, the suppressor potential of T(regs) appears to be strongly influenced by their compartmentalization. In myelodysplastic syndromes (MDS), immune deregulation and autoimmunity in the early stages might lead to ineffective hematopoiesis and bone marrow (BM) failure, whereas late-stage disease is characterized by the immune escape of the malignant clone. We show that these two stages of MDS are associated with differential T(reg) activity. Specifically, we found that in early stage MDS, compared with normal hematopoiesis and late stage MDS, T(regs) are dysfunctional and their BM homing through the CXCL12/CXCR4 axis is seriously impaired as a result of CXCR4 downregulation. Conversely, in late stage MDS, T(regs) are systemically and locally expanded and retain their function and migratory capacity. Moreover, T(reg) levels follow the disease course and are significantly reduced in treatment responding patients. Our findings indicate T(reg) involvement in the pathophysiology of MDS; defective suppressor function and BM trafficking of T(regs) may be important in the autoimmune process of early MDS, but increased T(reg) activity could favor leukemic clone progression in late stage disease.

Long-term bone marrow cultures (LTBMC) from essential thrombocythemia (ET) patients with or without JAK2617V>F mutation.

Approximately half of essential thrombocythemia (ET) patients and almost all with polycythemia vera (PV) bear the activating JAK2617V>F point mutation, which arises at the multipotent haemopoietic progenitor cell level. Although ET is mainly characterized by megacaryocyte proliferation, the cases that are positive for the JAK2617V>F mutation also show increased bone marrow cellularity and higher erythrocyte and granulocyte counts. After establishing short- and long-term bone marrow cultures we found that the frequency of committed haemopoietic progenitors in the bone marrow, was not increased in JAK2617V>F positive ET compared to the negative ones, whereas in long-term cultures (LTBMC) JAK2617V>F positive ET display a growth pattern more similar to that observed in LTBMC produced by PV marrow cells. Our data support the notion that JAK2617V>F positive ET and PV represents a continuum spectrum of alterations within the same disease.

Addition of cyclosporin-A to chemotherapy in secondary (post-MDS) AML in the elderly. A multicenter randomized trial of the Leukemia Working Group of the Hellenic Society of Hematology.

In elderly patients with secondary leukemia, poor therapeutic response and low overall survival have been attributed mainly to age and to the primary resistance of leukemic cells to chemotherapy. Modulation of resistance has been attempted in different studies, but the results have been contradictory. We conducted an open, randomized multicenter clinical trial involving patients more than 60 years old with secondary leukemia preceded by a myelodysplastic syndrome. The induction chemotherapy regimen included idarubicin, cytarabine, and etoposide (group A); randomization involved simultaneous administration of cyclosporin-A per os (group B). Fifty-five patients were evaluated, 26 in group A and 29 in group B. Overall complete remission was achieved in 40% of the patients, 27% vs 52% in groups A and B, respectively (p=0.01). Leukemia-free survival was more favorable in patients who received cyclosporin-A, 12 vs 7 months for groups B and A, respectively (p=0.03). In a follow up period of 30 months, 7 out of 55 patients (13%) were alive, 4 of whom were in complete remission. Five out of the 7 alive patients were randomized in group B and had received cyclosporin-A. Treatment failure was higher in group A [19 of 26 patients (73%)] than in group B with CsA [14 of 29 patients (48%)] (p<0.0001). Treatment-related toxicity/mortality was 13%. Modulation of drug resistance by CsA in elderly people suffering from secondary acute leukemia may improve the outcome of chemotherapy without increasing drug toxicity and treatment-related mortality.

Extramedullary plasmacytoma: report of two cases with uncommon presentation.

Lymph node infiltration by monoclonal plasma cells can occur either in aggressive forms of myeloma or may represent regional extension of extramedullary plasmacytomas, whereas lymph node plasmacytoma presenting as a solitary extramedullary plasmacytoma is very unusual. We report two cases of lymph node plasmacytomas without systemic disease diagnosed after surgical excision. Clinical remission was achieved after local radiotherapy although one patient relapsed with multifocal extramedullary plasmacytomas 20 months after radiotherapy.

Alterations of platelet function, number and indexes during acute pancreatitis.

BACKGROUND/AIMS: Acute pancreatitis constitutes a systemic inflammatory process which is often accompanied by thrombosis and bleeding disorders. The role of platelets in the pathophysiology of the disease has not been elucidated yet. The present study focuses on two successive end-points: (1) the activation of platelets during acute pancreatitis and (2) the alterations of platelet number and indexes between onset and remission of the disease, which reflect the bone marrow response. METHODS: A cohort of 54 patients with acute pancreatitis was enrolled. Cause and severity of the disease (APACHE II score) were estimated. Activated platelet ratio (APR) was estimated using flow cytometry at onset and remission. Platelet number (PLT), mean platelet volume (MPV), platelet large cell ratio (P-LCR) and platelet distribution width (PDW) were collected at onset and remission. RESULTS: The first end-point was reached in patient 14 as APR was found elevated at onset of acute pancreatitis (p = 0.01). The second end-point was fulfilled in patient 12 for MPV, P-LCR and PDW, which were found elevated at remission of the disease (p < 0.01) but not for PLT until the last patient (p = 0.34). CONCLUSION: Platelets are directly involved in the systemic inflammatory process of acute pancreatitis, which leads to consumption, compensated by an immediate bone marrow response.

Long-term remission of recalcitrant tumour-stage mycosis fungoides following chemotherapy with pegylated liposomal doxorubicin.

Advanced stage mycosis fungoides (MF) generally has a poor prognosis, and currently there is no standard treatment available. Here we report the case of a young woman with recalcitrant tumour-stage MF (T3, stage IIb) whose disease was unresponsive to several therapeutic modalities, but who has showed sustained clinical response to pegylated liposomal doxorubucin. No severe infectious complications have been observed. The use of this drug in tumour-stage MF should be investigated further.

[A study of esthetic harmony and balance of the facial soft tissue]. / Melete tes aisthetikes armonias kai isorrhopias ton malthakon iston tou prosopou.

Esthetics and harmony of the human body have been for ages sources of research and inspiration for scientists and artists. The face affects the appearance and determines, in a way, the limits in the evaluation of beauty influenced by various psychological, environmental or other factors. There are some studies dealing with facial proportions in which researchers are trying to establish some mathematic norms for the evaluation of the "esthetic face". In this paper, a study of some facial proportions is carried out, in photographs of female faces with three different expressions, seriousness, relaxed position and smile. Our findings show that smiling faces are significantly different from serious and relaxed faces in most of the proportions studied. The "Golden proportion" was closer to the proportions of the serious and relaxed faces than to the proportions of the smiling ones.